Kinky hair syndrome is a sex-linked recessively inherited copper metabilic disorder with severe neurodegenerative change and infant death. In 1962, Menkes and associates described five boys of a related pedigree with severe psychomotor retardation, seizures and widespread cerebral and cerebellar degeneration. In 1796,. Wesenberg and associates specified the raioligical characterization of the syndrome. Symmetrical metaphyseal spurring and diaphyseal periosteal reactio of thelong bones. anterior rib flaring, am malformed cerebral arterial system and subdural effusion. In 1972, Danks and associates found the disease to be associated with a defect of copper metabilism, confirmed by studies with labelled Cu. Authors experienced a case with characteristics clinical picture, and report cerebral and abdominal arteriorgrpahic changes and plain raidographic findings with brain CT, DSA and post-mortem angiogrpahy.